Cancer registries and rare cancers: quality of data, supplementary information RARECARE WP6 3rd meeting National Institute of Public Health Warsaw 25th March 2010 RARECARE data quality study on high priority rare cancers (Gemma Gatta, Annalisa Trama)

Objectives To verify the diagnostic accuracy To assess the completeness of incidence To verify the quality of follow-up To verify the availability of information on stage, treatment and place of treatment To improve the estimates of incidence and survival

Short list Mesothelioma Liver angiosarcoma Sarcomas Tumors of oral cavity SNC tumours Germ cell tumours Leukemia Endocrine tumours Primary prevention Diagnostic accuracy Secondary prevention Quality of care Data quality

Methods Revision of the clinical dossiers, pathologic reports filed at cancer registry offices Revision of the follow-up only for mesothelioma, angiosarcoma of the liver and CNS tumours Period of diagnosis:

33 CRs from 14 countries

Mesothelioma Issues for data quality Diagnostic accuracy Quality of follow-up

Mesothelioma, 3-year relative survival by registry EUROCARE-4 Registry that revised the data are marked with the red arrow

Mesothelioma revision The review focused on mesothelioma long term survivors ( ) of any sites (to verify the diagnostic accuracy and quality of follow-up) all cases with pleural cancers ≠ mesothelioma (to ascertain the completeness of incidence mesothelioma of the pleura)

Mesotelioma long survivors Cancer registries participating = 29 No. of mesothelioma long survivors revised = 551 (32% F; 68% M) Results of the morphology check 95% (524/551) were confirmed mesothelioma of (pleura, peritoneum, other male genital organs, unknown primary) 5% (27/551) were not mesothelioma. In details: 8 adk (colon, lung, breast, ovary) 6 neoplasm, NOS (lung, pleura, ovary) 3 sarcomas (lung, bone, thyroid gland) 1 lymphoma 9 (benign, border line, error)

Mesothelioma long survivors Results of the life status check 455/551 (83%) confirmed as mesothelioma long survivors 318 (70%) M; 137 (30%) F 68/551 (12%) were mesothelioma not long survivors 22 lost to follow-up 46 death date changed 23/551 (4%) were not mesothelioma long survivors

Pleura cancers ≠ mesothelioma Morphology before revision Freq.PercentCum. neoplasms, NOS adk squamous cell neoplasms sarcomas thymoma hematopoietic Total Cancer registries participating = 29 No. of cases revised = 681 (58% M, 42% F)

Pleural cancers ≠ mesothelioma 465/681 (68%) pleura cancers 323 = neoplasms, NOS 47 = mesotheliomas 34 = sarcomas 56 = adk 5 = squamous cell 216/681 (32%) not pleura cancers 87 = adk (digestive, respiratory, female genital, urinary, unknown primary) 67 = neoplasms, NOS (digestive tract, respiratory system, female gen organs, unknown primary) 26 = hematopoietic 9 = squamous cell (respiratory, urinary tract) 9 = sarcomi (respiratory, skin, soft tissue, unknown primary) 55 = benign 2 = records not reviewed Results of the morphology check

Malignant digestive endocrine tumour (MDET) Issues for data quality Diagnostic accuracy (difficulties in distinguish tumors with different prognosis) Undifferentiated small-cell MDET (8041/3) and (8042/3) Well-differentiated MDET (8150/3), (8151/3), (8153/3), (8155/3), (8152/3), (8246/3), (8240/3, 8241/3, 8243/3, 8244/3) Behaviour (carcinoids)

MDET The review focused on undifferentiated (8020/3) and anaplastic (8021/3) carcinomas of the digestive tract (C15 to C25) ( to find small cell MDET) all carcinoids ( ) of the digestive tract (C15 to C25) (to verify the behaviours) Criteria for defining the behaviour of carcinoids: Invasion of the muscularis propria Dimension of the tumour

Well differentiated benign and bordeline ET Well differentiated endocrine carcinoma Undiff endocrine carcinoma DifferentiationWell differentiated Undifferentiated AngioinvasionNoPossible SizeStomach, Small intestine: < 1cm Appendix, colon, rectum: < 2 cm Pancreas : < 2 cm Stomach, Small intestine: >1 cm Appendix, colon, rectum: > 2 cm Pancreas : >2 cm Mitotic Index< 2< 22 to 10> 10 Prol index< 2 %2 to 15 %> 15 % Local invasionDigestive tumour: mucosae/submucosae Pancreas: intra-pancreatic Digestive tumour: > Muscularis propria Appendix: invasion of the visceral peritoneum Pancreas: extra- pancreatic extension MetastasesnoPossible Behavior: /1 /3 /3

Carcinoids (behavior) Cancer registries participating = 21 No. of cases revised = 1672 Information for defining the behavior: available for ONLY 223 cases Behavior defined only if both dimension of the tumor and local invasion were available

Topography Behavior /1/3 NAdeleted Total esophagus00505 stomach small intestine colon appendix recto-sigmoid junction rectum anus and anal canal10506 liver00101 gallbladder00707 other, unspec parts of biliar tract30508 pancreas thymus10001 Total ,672 Carcinoids (behaviour): results

Undifferentiated and anaplastic carcinomas Cancer registries participating = 26 No. of cases revised = 844 Freq.% undifferentiated carcinoma small cell tumors70.83 epithelial neoplasms, NOS91.07 squamous cell40.47 adk sarcomas20.24 lymphoma10.12 info not avail not malignant20.24 Total Results of the morphology check

Central Nervous System tumours Issues for data quality Diagnostic accuracy Quality of follow-up

from 27% to 16% >20% (Finland, Iceland, Norway, Ireland, Wales, Austria, Belgium, Germany, Switzerland, Portugal) source: Sant et al, EJC, 2008 CNS tumours 5-year relative survival

CNS tumours revision The review focused on Long-term survivors with a diagnosis of unspecified morphology codes (8000, 8001, 8010) (to verify the diagnostic accuracy and quality of follow-up) Cases with diagnosis of Glioma NOS (9380) microscopically verified (to verify the diagnostic accuracy for tumours with treatment options)

CNS tumours long survivors Cancer registries participating = 22 No. of brain cancers long survivors revised = 705 (53% F; 47% M) Results of the morphology check 93% (653/705) were confirmed brain tumours 544 = neoplasms NOS 44 = astrocitomi 6 = oligodendroglial 6 = non glial/embryonal tumurs 2 = ependimal tumours 1 = sarcoma 47 = not malignant 3 = epithelial neoplasms, NOS 5% (27/551) were not mesothelioma. In details: 8 adk (colon, lung, breast, ovary) 6 neoplasm, NOS (lung, pleura, ovary) 3 sarcomas (lung, bone, thyroid gland) 1 lymphoma 9 (benign, border line, error)

CNS tumours long survivors Results of the morphology check 7% (52/705) were not brain tumours 17 = meningiomas 4 = sarcomas 3 = neoplasms, NOS 2 = lung tumours ( 1epithelial and 1 squamous cell neoplasm) 2 = breast adk 2 = lymphomas 2 = ependimal tumours 1 = skin melanoma 1 = endocrine glands germinoma 1= spinal cord astrocitoma 11 = not malignant 8 = information not available

CNS tumours long survivors Results of the life status check 343/705 (49%) confirmed as real long survivors Real brain tumors survivors 282/705 (40%) Not brain tumours long survivors 61/705 (9%) 337/705 (48%) were brain tumours not long survivors 124 lost to follow-up 213 death date changed For 25 cases (3%) the information on the follow-up was missing. It has to be verfied with CRs

Glioma, NOS Cancer registries participating = 21 No. of cases revised = 472 (55% M, 45% F) Morphology after the revisionFreq.% glioma malignant astrocytic tumors oligodendroglial51.06 non glial/embryonal tumors30.64 ependimal tumors10.21 neoplasms, NOS40.85 sarcoma20.42 not malignant81.69 Total cases of brain tumours will contribute to modify incidence and survival of the second layer entities

Gonadal germ cell tumours Issues for data quality Diagnostic accuracy for tumors with treatment options (% of morphology codes, NOS)

Gonadal germ cell tumours The review focused on: morphology NOS ( ) cases of the testis and of the ovary. ONLY microscopically verified cases

Gonadal germ cell tumours (1) Cancer registries participating = 25 No. of cases revised = 1829 Results of the morphology check 89% (1629) were confirmed unspecified morphology of female and male genital organs 2.3% (41) = non epithelial tumors ovary/testis 23 = germ cell tumours (19 testis; 4 ovary) 12 = sex cord tumors of the ovary 6 = malignant/immature teratomas (2 ovary, 4 testis)

Gonadal germ cell tumours (2) Results of the morphology check (2) 5.96% (109) = adk (stomach, breast, 105 ovary, prostate, 1 testis) 1.53% (28) = other epithel neoplasms, NOS of the ovary and testis 0.16% (3) = squamous cell ca ovary 0.16% (3) = mixed epithel/mesench tumor of the ovary 0.11% (2) = sarcomas (soft tissue and ovary) 0.05% (1) = transitional cell carcinomas of the bladder 0.05% (1) = mesothelioma 0.05% (1) = ependimoma of the brain 0.05% (1) = lymphoma 0.44% (8) = benign tumours 0.11% (2) = deleted The revision identified 23/1829 (1.2%) germ cell tumors

Ovary and Testis

Liver angiosarcoma With morphology different from colangio/hepatocellularcarcinoma, hepatoblastoma. Sarcoma NOS Angiosarcoma long survisors (>1yr)

Angiosarcoma, long survivors 4 CRs identified long survivors 13 long survivors 6 long survivors (2m, 4f) (range: 16 months-11years) 5 cases were lost to follow-up, 2 survived <1years

Liver, sarcoma NOS 8 CRs identified sarcoma NOS (18 cases checked)

Epithelial tumor of the liver, morphology not typical 1306 cases checked (29 CRs) 1134 cases of liver (87%) None angiosarcoma

Epithelial tumor of the liver, morphology not typical 1306 cases checked (29 CRs) 172 cases ≠ of liver (13%)

Sarcomas Pathological diagnosis is not easy Delay in diagnosis and treatment Checks of Sarcoma NOS or descriptive codes (8800, )

% Sarcoma NOS by registry by registry Registry that verified the data are marked with the red arrow

Sarcomas

Sarcomas Sarcomas Sarcomas different codesNo sarcomas

CRs Sarcoma NOS before and after the revision

Leukaemia Two major types: typical and atypical CML with different prognosis Revision of the unspecified codes of leukaemia and CML, NOS (9800-1, 9820, 9860, 9863)

CML, NOS

Other leukaemia/lymphoma (55 cases)

Leukaemia, NOS

Other leukaemia/lymphoma (219 cases, 15%)

% of leukaemia NOS by registry (average 6%)

Tumours of oral cavity Issues for data quality Diagnostic accuracy Morphology: neoplasms, NOS and carcinoma NOS (ICD-O 8000, 8001, 8010, 8011) Topography: tongue overlapping lesion (C2.8) and palate, NOS (C5.9) Both subsites are in oropharynx Morphology NOS are in the layer 1 entity

Tumours of oral cavity revision The review focused on morphology codes 8000, 8001, 8010, 8011 (carcinoma NOS) of the oral cavity (to verify the diagnostic accuracy) C02.0-C02.3, C02.9, C03.0-C05.0, C06.0-C06.9 dorsal and ventral surface of the tongue, border of the tongue, anterior 2/3 of tongue,.gum, floor of mouth, …mouth NOS C01.9, C02.4, C02.8, C05.1-C05.2, C05.9, C09.0-C10.3, C , C14.2 base of tongue, lingual tonsil, tongue overlapping, soft palate, uvula, palate, NOS,…Waldeyer ring. unspecific site codes C02.8 (overlapping lesion of the tongue) and C05.9 (palate, NOS) (to distinguish between oral cavity and oropharynx) 5-year survival: oropharynx = 37% oral cavity = 59% Oral cavity Oropharynx

Carcinoma, NOS Cancer registries participating = 26 No. of cases revised = 555 (68% M, 32% F) Freq.% carcinoma, NOS squamous cell adenocarcinoma50.9 sarcomas10.2 Not in incidence any more 50.9 Total Results of the morphology check

Unspecified sites Cancer registries participating = 22 No. of cases revised = 388 (71% M, 29% F) Results of the topography check Freq.% unspecified sites oral cavity oropharynx nasopharynx10.26 hypopharynx10.26 erased20.52 Total388100

Some conclusions (1) Mesothelioma, angiosarcoma, CNS neoplasms NAS long survivors Can we further describe these cases? Can we suggest recommendations to registries? Pleura and CNS epithelial tumours Can we suggest recommendations to registries

Some conclusions (2) MDET Can we accept the criteria for define the invasion for carcinoids? Can we improve the diagnosis? Sarcomi GIST Leukaemia Underestimation of CML incidence Evolution of the lymphoma and leukaemia classification Others?